Volume 78 (2012), No. 6/2012(December)
The online-version will be updated before the print-version of this Journal is published. Upon request we will send the password and user name by e-mail. The online-service is only available for subscribers of the print-version, if proof of purchase is submitted.
The use of the online-version will be charged with an extra fee (additional to the subscription of the print-version). The service can be used until December 31st of the year of subscription.
|
| Price of the complete print-issue: 30.00$ |
Add to Cart
|
Original
The association between coronary artery calcification progression and loss of bone density in non-dialyzed CKD patients
Renato Watanabe, Marcelo M. Lemos, Aluizio B. Carvalho, Carlos E. Rochitte, Raul D. Santos, Sérgio A. Draibe and Maria Eugênia F. Canziani
Page No. 425
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (425-431)
The association between coronary artery calcification progression and loss of bone density in non-dialyzed CKD patients
Renato Watanabe1, Marcelo M. Lemos1, Aluizio B. Carvalho1, Carlos E. Rochitte2, Raul D. Santos2, Sérgio A. Draibe1 and Maria Eugênia Canziani1
1Department of Internal Medicine, Division of Nephrology, Federal University of São Paulo, and 2Heart Institute (InCor), University of São Paulo, Medical School Hospital, São Paulo, Brazil
Background: Coronary artery calcification (CAC) and low bone density are coexisting deleterious conditions commonly shared by chronic kidney disease (CKD) patients. In the present study, we aimed to investigate whether the progression of CAC was associated with overtime reduction in bone density in non-dialyzed CKD patients. Methods: This is a prospective study of 24 months including 72 non-dialyzed CKD patients Stages 2 – 4 (age 57.6 ± 10.3 years, 62% male, 22% diabetics). CAC and vertebral bone density (VBD) were measured by computed tomography. Results: At baseline, 46% of the patients had CAC (calcified group) and calcification was not identified in 54% of the patients (non-calcified group). The calcified group was older, predominantly male, and had lower VBD in comparison to non-calcified group. CAC progression was observed only in the calcified group (91% of the patients increased calcium score). The multiple regression analysis revealed loss of VBD as the independent determinant of CAC progression in these patients. Conclusions: CAC progression was associated with loss of VBD in non-dialyzed CKD patients.Correspondence to:
Maria Eugênia, MD, PhD
Fernandes Canziani Rua Pedro de Toledo 282,
CEP 04039-000, São Paulo – SP, Brazil
Email: [email protected]
Original
25-hydroxyvitamin D deficiency is associated with an increased risk of metabolic syndrome in patients with non-diabetic chronic kidney disease
Stephanie Seiki, Michel Chonchol, Alfred K. Cheung, James S. Kaufman, Tom Greene, William L. Roberts, Gerard Smits, Jessica Kendrick and the HOST Investigators
Page No. 432
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (432-441)
25-hydroxyvitamin D deficiency is associated with an increased risk of metabolic syndrome in patients with non-diabetic chronic kidney disease
Stephanie Seiki1, Michel Chonchol1, Alfred K. Cheung2,3, James S. Kaufman4, Tom Greene5,6, William L. Roberts7, Gerard Smits1, Jessica Kendrick1,8 and the HOST Investigators
1Division of Renal Diseases and Hypertension, University of Colorado School of Medicine, Aurora, CO, 2Renal Section, Medical Service, Veterans Affairs Salt Lake City Healthcare System, 3Division of Nephrology & Hypertension, University of Utah, Salt Lake City, UT, 4Renal Section, Medical Service, Veterans Affairs Boston Healthcare System and Boston University School of Medicine, Boston, MA, 5Research Service, Veterans Affairs Salt Lake City Healthcare System, 6Division of Epidemiology, 7Department of Pathology, University of Utah, Salt Lake City, UT and 8Denver Health Medical Center, Denver, CO, USA
Background: Patients with chronic kidney disease (CKD) not requiring dialysis have a high prevalence of 25-hydroxyvitamin D (25(OH)D) deficiency but the relationship between 25(OH)D levels and metabolic syndrome is unknown in this population. Methods: This study analyzed stored plasma samples from 495 non-diabetic subjects with severe kidney disease, not yet on dialysis, who participated in the homocysteine in kidney and end stage renal disease study. Metabolic syndrome was defined as the presence of all three of the following: (1) Serum triglycerides ≥ 150 mg/dl or drug treatment for hypertriglyceridemia; (2) serum high density lipoprotein-cholesterol (HDL-C) < 50 mg/dl for women or < 40 mg/dl for men or drug treatment for dyslipidemia; and (3) blood pressure ≥ 130/85 mmHg or drug treatment for hypertension. Multivariate logistic regression models were used to evaluate the cross-sectional association between plasma 25(OH)D levels and metabolic syndrome. Results: The prevalence of metabolic syndrome increased as 25(OH)D levels declined, with the highest prevalence in participants with 25(OH)D levels < 20 ng/ ml. Participants with 25(OH)D levels < 20 ng/ml had a significantly increased risk of metabolic syndrome compared to subjects with levels > 30 ng/ml after adjustment for multiple confounders (OR 2.25, 95% CI 1.25 – 4.07). Plasma 25(OH)D levels were inversely associated with diastolic blood pressure (R = –0.10, p = 0.029) and serum triglyceride levels (R = –0.14, p = 0.002). Conclusion: 25(OH)D deficiency is strongly associated with an increased risk of metabolic syndrome in non-diabetic patients with severe CKD not yet on dialysis, independent of cardiometabolic risk factors and other important regulators of mineral metabolism.Correspondence to:
Jessica Kendrick, MD
Assistant Professor
Division of Renal Diseases and Hypertension
University of Colorado School of Medicine
Denver Health Medical Center
660 Bannock Street Mail Code 4000
Denver, CO 80204, USA
Email: [email protected]
Original
Patients with diabetes mellitus Type 2 and hypomagnesemia may have enhanced glomerular filtration via hypocalcemia
Phuong-Chi T. Pham, Phuong-Mai T. Pham and Phuong-Thu T. Pham
Page No. 442
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (442-448)
Patients with diabetes mellitus Type 2 and hypomagnesemia may have enhanced glomerular filtration via hypocalcemia
Phuong-Chi T. Pham1, Phuong-Mai T. Pham2 and Phuong-Thu T. Pham3
1UCLA-Olive View Medical Center, Division of Nephrology and Hypertension, Sylmar, 2VA Greater Los Angeles Healthcare System, Sepulveda Ambulatory Care Center, Department of Internal Medicine, North Hills, and 3David Geffen School of Medicine at UCLA, Kidney and Pancreas Transplant, Los Angeles, CA, USA
Introduction: Hypomagnesemia and glomerular hyperfiltration are commonly observed in patients with diabetes mellitus Type 2 (DM2). In the current study, we examined the relationship between hypomagnesemia and glomerular filtration rates in DM2 patients. Materials and methods: Data were obtained for DM2 patients without documented kidney disease seen at UCLAOlive View Medical Center during January through March 2001. Data for hemoglobin, hemoglobin A1C (HbA1C), routine electrolytes, lipid profiles, urinalyses, history of hypertension, and pharmacy profiles were retrieved. Estimation of glomerular filtration rate (eGFR) was based on the CKD-epi formula. Multivariate analyses were performed to determine the correlations between eGFR and clinical factors including age, gender, history of hypertension, the use of diuretics, renin angiotensin system (RAS) inhibitors, acetylsalicylic acid, and statins, serum calcium, magnesium, hemoglobin, HbA1C lipid profile, and degree of proteinuria. Results: 550 patients (54% females) with mean age 57.5 ± 11.0 years and eGFR 95.7 ± 14.8 ml/min/1.73 m2 were included. Multivariate analysis revealed negative correlations with eGFR for age (Pearson-correlationcoefficient: –0.7, p < 0.0001), hypertension (–0.32, p < 0.0001), magnesium (–0.21, p < 0.0001), calcium (–0.13, p = 0.009), proteinuria (–0.17, p < 0.0001), and the use of RAS inhibitors (–0.21, p < 0.0001), and diuretics (–0.24, p < 0.0001) and a positive correlation for HbA1C (0.28, p < 0.0001). Further analysis of the interaction between serum magnesium and calcium, defined as magnesium × calcium (Mg × Ca), revealed a more significant correlation with eGFR than either cation alone (–0.24, p < 0.0001). Conclusions: Serum magnesium, calcium, and (Mg × Ca) all had significant negative correlations with eGFR. In particular, (Mg × Ca) had the strongest correlation with eGFR.Correspondence to:
Phuong-Chi T. Pham
UCLA-Olive View Medical Center
Division of Nephrology and Hypertension
14445 Olive View Drive, 2B-182
Sylmar, CA 91342, USA
Email: [email protected]
Original
Differences in community, hospital and intensive care unit-acquired acute kidney injury: observational study in a nephrology service of a developing country
Elizabeth F. Daher, Geraldo B. Silva Junior, Silvia Q. Santos, Carla Camila R. Bezerra, Elton J.B. Diniz, Rafael S.A. Lima, Célio A. Babosa, Antonio Augusto C. Guimarães, Rosa M.S. Mota, Krasnalhia Lívia S. Abreu and Alexandre B. Libório
Page No. 449
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (449-455)
Differences in community, hospital and intensive care unit-acquired acute kidney injury: observational study in a nephrology service of a developing country
Elizabeth F. Daher1,2, Geraldo B. Silva Junior2,3, Silvia Q. Santos1, Carla Camila R. Bezerra1, Elton J.B. Diniz1, Rafael S.A. Lima4, Célio A. Babosa1, Antonio Augusto C. Guimarães1, Rosa M.S. Mota5, Krasnalhia Lívia S. Abreu2 and Alexandre B. Libório1,3
1Division of Nephrology, General Hospital of Fortaleza, 2Department of Internal Medicine, School of Medicine, Federal University of Ceará, 3School of Medicine, University of Fortaleza. Fortaleza, Ceará, 4Division of Nephrology, University of São Paulo, Ribeirão Preto, São Paulo, and 5Department of Statistics, Center of Science, Federal University of Ceará. Fortaleza, Ceará, Brazil
Background: Acute kidney injury (AKI) complicates more than 7% of all in-hospital patients. The aim of this study is to investigate the differences in community, hospital and intensive care unit-acquired AKI in patients undergoing nephrology consultation in a tertiary hospital in a developing country. Methods: An observational cohort study of all patients with AKI admitted to the General Hospital of Fortaleza, Brazil was conducted. RIFLE criteria were used to classify the patients and to assess their association with death. Univariate and multivariate analyses were performed to investigate the factors associated with death. Results: Of 491 AKI patients undergoing nephrology consultation, the mean age was 55.2 ± 22.9 years. Community-acquired AKI was observed in 55% of cases, general ward-acquired in 29% and ICU-acquired in 15.3%. Late Nephrology consultation was observed, and the great majority of patients had “Failure” classification (90%) according to RIFLE criteria. Intermittent hemodialysis was required in 68% of cases. The overall in-hospital mortality was 23%. The in-hospital mortality was higher in ICU-acquired AKI (33.6%). Community acquired AKI had a higher mortality than general ward-acquired AKI (23% vs. 11.6%, p = 0.001). Risk factors for death were infection (OR = 2.0, p = 0.003), neoplasms (OR = 1.89, p = 0.042), community acquired-AKI (OR = 1.27, p = 0.003), ICU acquired-AKI (OR = 2.76, p < 0.0001) and need for renal replacement therapy (OR = 2.64, p < 0.001). Conclusions: AKI is a frequent and frequently fatal condition. Mortality was higher in community and ICU-acquired than hospital ward-acquired AKI.Correspondence to:
Dr. Alexandre Braga Libório and Elizabeth Daher
Rua Vicente Linhares, nº 1198.
CEP 60135-270, Fortaleza, CE, Brazil
Email: [email protected]
Original
Clinical and pathological analysis of hepatitis B virus-related membranous nephropathy and idiopathic membranous nephropathy
Ping Li, Ri-bao Wei, Li Tang, Jie Wu, Xue-guang Zhang and Xiang-mei Chen
Page No. 456
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (456-464)
Clinical and pathological analysis of hepatitis B virus-related membranous nephropathy and idiopathic membranous nephropathy
Ping Li*, Ri-bao Wei*, Li Tang, Jie Wu, Xue-guang Zhang and Xiang-mei Chen
Nephropathy of Chinese PLA General Hospital, State Discipline and Key Laboratory of Kidney Disease (Chinese PLA General Hospital 2011DAV00088), Beijing, China
*These authors contributed equally to this work as the first author.
Objective: The Hepatitis B virus infection rate is high in the Chinese population. The implications of HBV infection are widely recognized, and membranous glomerulonephritis is the most common renal lesion associated with HBV infection. We compared the clinicopathologic features of 119 HBV-related membranous nephropathy (HBV-MN) and 143 idiopathic membranous nephropathy (IMN) patients to identify those factors that facilitate their discrimination. Methods: Cohort analysis of demographic information, clinical manifestations, laboratory parameters, renal pathology and prognostic features of the two groups. Results: Most HBV-MN patients were young or middle-aged; the onset age in the HBVMN group was younger than the IMN group (p < 0.05). A male predominance was found in both groups. And the two groups both presented with heavy proteinuria or nephrotic syndrome. In contrast to IMN patients, the HBV-MN group was presented with a high occurrence of microscopic hematuria (73.95 vs. 35.66%) and renal insufficiency (47.06 vs. 24.48%). Plasma complement C3 and C4 in HBV-MN patients were significantly lower than in IMN patients (p < 0.05). The hyperlipidemia was more severe in IMN patients (p < 0.05). The occurrences of segmental glomerular damage, mesangial cell proliferation and tubulointerstitial damage were more common in the HBV-MN group (p < 0.05). Immunofluorescence staining of polyclonal immunoglobulin and polytypic complement immunoglobulin were more frequent in the HBV-MN group. The followup data showed there were no statistic differences in the prognosis between HBV-MN and IMN. Conclusion: HBV-MN patients commonly showed nephrotic syndrome accompanied with renal and hepatic dysfunction which was different from IMN patients. The primary pathological feature of HBVMN was atypical membranous nephropathy, which is usually associated with the inflammatory changes in HBV infection. The renal survival rates did not differ between HBVMN patients and IMN patients.Correspondence to:
Xiangmei Chen, MD/PhD
State discipline and Key Laboratory of Kidney
Disease (Chinese PLA General Hospital)
Beijing, 100853, PR China
Email: [email protected]
Original
Association of IgG co-deposition with serum levels of galactose-deficient IgA1 in pediatric IgA nephropathy
T. Matthew Eison, M. Colleen Hastings, Zina Moldoveanu, John T. Sanders, Lillian Gaber, Patrick D. Walker, Keith K. Lau6, Bruce A. Julian, Jan Novak and Robert J. Wyatt
Page No. 465
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (465-469)
Association of IgG co-deposition with serum levels of galactose-deficient IgA1 in pediatric IgA nephropathy
T. Matthew Eison1, M. Colleen Hastings1, Zina Moldoveanu2, John T. Sanders3, Lillian Gaber4, Patrick D. Walker5, Keith K. Lau6, Bruce A. Julian2, Jan Novak2 and Robert J. Wyatt1
1University of Tennessee Health Science Center and Children’s Foundation Research Center at Le Bonheur Children’s Hospital, Memphis, TN, 2University of Alabama at Birmingham, Birmingham, AL, 3Sanford Children’s Hospital, Sioux Falls, SD, 4Department of Pathology, The Methodist Hospital, Houston, TX, 5Nephropathology Associates, Little Rock, AR, USA, and
6McMaster University, Hamilton, Ontario, Canada
Objective: To determine whether the absence of mesangial IgG deposits is associated with the absence of elevated blood levels of galactose-deficient IgA1 (Gd-IgA1) in pediatric patients with IgA nephropathy (IgAN). Design and methods: Serum Gd-IgA1 levels were determined by ELISA using an N-acetylgalactosamine-specific lectin from Helix aspersa. Levels of Gd-IgA1 above the 90th percentile for healthy pediatric controls were considered to be elevated. Renal biopsy samples were examined by immunofluorescence for presence and intensity of staining for IgA, IgG, IgM, C3 and C1q and by light microscopy for histological changes. Findings were graded by a single pathologist (L. Gaber) at UTHSC until 2007 and by NephropathTM (Little Rock, AR, USA) thereafter. Staining for the mesangial deposits was considered negative when intensity was trace or less, and positive at greater intensity. Fisher’s exact test was used to determine significance of 2 × 2 tables. Results: Serum samples were obtained from 30 patients with IgAN diagnosed before age 18 years. Male:female ratio was 2.3:1. Twenty were Caucasian and 10 were African-American. Blood was obtained within 3 months of biopsy (incident cases) for 12, while 18 provided blood > 3 months after biopsy (prevalent cases). Serum Gd-IgA1 level was elevated in 23 (77%) of cases and 20 (67%) had a biopsy positive for IgG. Of those 20 patients, 18 (90%) had an elevated serum Gd-IgA1 level, whereas 5 (50%) of patients with biopsies without IgG had a normal serum Gd-IgA1 level (p = 0.026). Summary: In this small study we found a weak association between the absence of IgG in the biopsy and normal serum Gd-IgA1 level.Correspondence to:
Dr. Robert J. Wyatt, MD, MS
Department of Pediatrics, Nephrology Division
Le Bonheur Children’s Hospital
50 North Dunlap, Research Tower, Room 520 R
Memphis, TN, USA
Email: [email protected]
Original
Relationship between glomerulomegaly and clinicopathologic findings in IgA nephropathy
Won Seok Jang, Kyung-Hwan Jeong, Joo-Young Moon, Sang Ho Lee, Joo Hee Cho, Tae-Won Lee, Yong-Koo Park, Byoung Soo Cho and Chun-Gyoo Ihm
Page No. 470
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (470-477)
Relationship between glomerulomegaly and clinicopathologic findings in IgA nephropathy
Won Seok Jang1*, Kyung-Hwan Jeong1*, Joo-Young Moon1, Sang Ho Lee1, Joo Hee Cho1, Tae-Won Lee1, Yong-Koo Park2, Byoung Soo Cho3 and Chun-Gyoo Ihm1
1Departments of Nephrology, 2Pathology and 3Pediatrics, Kyung Hee University School of Medicine, Seoul, Korea
*Both authors have contributed equally to the manuscript and are acknowledged as co-first authors.
Background: Large glomeruli are a common finding in the early stages of progressive renal disease. We studied the relationship between glomerular surface area (GSA) and clinicopathologic features of IgA nephropathy (IgAN), including renal outcome, to better understand the role of GSA in IgAN. Methods: We analyzed renal biopsy specimens and clinical information from 34 patients with IgAN. Mean and maximal GSA were determined using a computed imaging analyzer. Results: Mean GSA was 16,811 ±4,671 μ2 in IgAN patients (n = 34). When we analyzed various clinical parameters of IgAN patients, there were significant correlations between mean or maximal GSA and age, body mass index (BMI), systolic and diastolic blood pressure, estimated glomerular filtration rate (eGFR), and pathologic findings including H.S. Lee’ grades, interstitial fibrosis, and tubular atrophy. GSA did not show any relationship with the degree of hematuria and proteinuria. By multivariate regression analysis of age, BMI, blood pressure, H.S. Lee’ grades, and eGFR as independent variables, mean GSA was associated with H.S Lee’ grades and initial eGFR. The results for maximal GSA were the same as those for mean GSA. When we divided IgAN patients according to their mean levels of GSA, the group with larger GSA had higher blood pressure and H.S. Lee’ grades and lower initial and final eGFR. More patients in the larger GSA group showed the decline in eGFR of more than 15 ml/min/1.73 m2 during the followup period compared with the smaller group. Conclusion: These results suggest that glomerular size, estimated by measuring GSA, is related to pathologic findings and renal function in IgAN. However, further investigation is required to determine if GSA can be used as a prognostic indicator of IgAN.Correspondence to:
Chun-Gyoo Ihm, MD, PhD
Departments of Nephrology
Kyung Hee University School of Medicine 1
Hoegi-dong, Dongdaemun-ku, Seoul, 130-701, Korea
Email: [email protected]
In-Depth Review
Acute kidney injury in the pregnant patient
Rosemary Nwoko, Darko Plecas and Vesna D. Garovic
Page No. 478
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (478-486)
Acute kidney injury in the pregnant patient
Rosemary Nwoko1, Darko Plecas2 and Vesna D. Garovic1
1Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN USA, and 2University Hospital for Obstetrics and Gynecology, School of Medicine, University of Belgrade, Serbia
Acute kidney injury (AKI) is costly and is associated with increased mortality and morbidity. An understanding of the renal physiologic changes that occur during pregnancy is essential for proper evaluation, diagnosis, and management of AKI. As in the general population, AKI can occur from prerenal, intrinsic, and post-renal causes. Major causes of pre-renal azotemia include hyperemesis gravidarum and uterine hemorrhage in the setting of placental abruption. Intrinsic etiologies include infections from acute pyelonephritis and septic abortion, bilateral cortical necrosis, and acute tubular necrosis. Particular attention should be paid to specific conditions that lead to AKI during the second and third trimesters, such as preeclampsia, HELLP syndrome, acute fatty liver of pregnancy, and TTP-HUS. For each of these disorders, delivery of the fetus is the recommended therapeutic option, with additional therapies indicated for each specific disease entity. An understanding of the various etiologies of AKI in the pregnant patient is key to the appropriate clinical management, prevention of adverse maternal outcomes, and safe delivery of the fetus. In pregnant women with pre-existing kidney disease, the degree of renal dysfunction is the major determining factor of pregnancy outcomes, which may further be complicated by a prior history of hypertension.Correspondence to:
Vesna D. Garovic, MD
200 1st Street SW
Rochester, MN 55905, USA
Email: [email protected]
Nephrology Education
Development of Wernicke’s encephalopathy during initiation of hemodialysis in an elderly non-alcoholic patient
Hiroshi Kimura, Kazuhito Takeda, Yoshiharu Muto, Hideyuki Mukai, Masahide Furusho, Satsuki Nakashita, Shuhei Miura, Atsuhiro Maeda and Kazuhiko Tsuruya
Page No. 487
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (487-491)
Development of Wernicke’s encephalopathy during initiation of hemodialysis in an elderly non-alcoholic patient
Hiroshi Kimura1, Kazuhito Takeda1, Yoshiharu Muto1, Hideyuki Mukai1, Masahide Furusho1, Satsuki Nakashita1, Shuhei Miura1, Atsuhiro Maeda1 and Kazuhiko Tsuruya2
1Department of Nephrology and Kidney Center, Iizuka Hospital, Iizuka City, and 2Department of Integrated Therapy for Chronic Kidney Disease, Kyushu University, Fukuoka City, Japan
A 79-year-old man with chronic renal failure developed general fatigue and loss of appetite. He was diagnosed with endstage renal disease and was started on hemodialysis (HD). The symptoms improved immediately, but the mental status deteriorated gradually, reaching Glasgow Coma Scale (GCS) 5. Computed tomography showed no significant intracranial lesion, but magnetic resonance images showed symmetric high-intensity changes in the periaqueductal area, suggestive of Wernicke’s encephalopathy (WE). He was immediately treated with intravenous infusion of thiamine. Five days later, the mental status level improved up to GCS 14, and the above MRI findings disappeared. To our knowledge, this is the first report describing the clinical outcome of a non-alcoholic patient who developed WE during initiation of HD. WE should be suspected in patients who are on chronic HD as well as those on initiation of HD with unexplained neurological abnormalities.Correspondence to:
Hiroshi Kimura, MD
Department of Nephrology and Kidney Center
Iizuka Hospital
3-83, Yoshio-Cho, Iizuka City, Fukuoka 820-8505, Japan
Email: [email protected]
Nephrology Education
Bartter syndrome Type III and congenital anomalies of the kidney and urinary tract: an antenatal presentation
Rik Westland, Wilfried W. Hack, Henricus J.R. van der Horst, Lukas B. Uittenbogaard, Johanna M. van Hagen, Paul van der Valk, Erik J. Kamsteeg, Lambert P. van den Heuvel and Joanna A.E. van Wijk
Page No. 492
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (492-496)
Bartter syndrome Type III and congenital anomalies of the kidney and urinary tract: an antenatal presentation
Rik Westland1, Wilfried W. Hack2, Henricus J.R. van der Horst3, Lukas B. Uittenbogaard4, Johanna M. van Hagen5, Paul van der Valk6, Erik J. Kamsteeg7, Lambert P. van den Heuvel8 and Joanna A.E. van Wijk1
1Department of Pediatric Nephrology, VU University Medical Center, Amsterdam, The Netherlands, 2Department of Pediatrics, Medical Center Alkmaar, Alkmaar, The Netherlands, 3Department of Pediatric Urology, 4Department of Obstetrics and Gynecology, 5Department of Clinical Genetics, 6Department of Pathology, VU University Medical Center, Amsterdam, The Netherlands, 7Radboud University Nijmegen Medical Center, Department of Human Genetics, Nijmegen, The Netherlands, and 8Radboud University Nijmegen Medical Center and Catholic University of Leuven, Department of Pediatric Nephrology, Nijmegen, The Netherlands, and Leuven, Belgium
Bartter syndrome encompasses a variety of inheritable renal tubular transport disorders characterized by hypokalemia and hypochloremic metabolic alkalosis. Bartter syndrome Type III is caused by genetic alterations in the chloride channel kidney B (CLCNKB) gene and often presents in the first 2 years of life, known as classic Bartter syndrome. However, in rare cases Bartter syndrome Type III has an antenatal presentation with polyhydramnios, premature delivery and severe dehydration in the first weeks of life. Associations between congenital anomalies of the kidney and urinary tract and Bartter syndrome are extremely rare. This case report presents a girl with Bartter syndrome Type III due to a homozygous CLCNKB mutation and bilateral congenital anomalies of the kidney and urinary tract. In addition, we describe the antenatal presentation as well as its perinatal management.Correspondence to:
Rik Westland, MD
Department of Pediatric Nephrology
VU University Medical Center
PO Box 7057
1007 MB Amsterdam, The Netherlands
Email: [email protected]
Nephrology Education
Schnitzler syndrome complicated by membranous nephropathy
Yoichi Iwafuchi, Takashi Morita, Kanako Hata, Akemi Nakamura and Shigeru Miyazaki
Page No. 497
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (497-500)
Schnitzler syndrome complicated by membranous nephropathy
Yoichi Iwafuchi1, Takashi Morita2, Kanako Hata1, Akemi Nakamura3 and Shigeru Miyazaki4
1Department of Internal Medicine, Koseiren Sanjo General Hospital, Sanjo, 2Department of Pathology, Shinrakuen Hospital, Niigata, 3Department of Dermatology, Koseiren Sanjo General Hospital, Sanjo, and 4Department of Internal Medicine, Kidney Center, Shinrakuen Hospital, Niigata, Japan
Schnitzler syndrome is a rare clinical entity characterized by the association of chronic urticarial rash and monoclonal immunoglobulin M gammopathy. A 62-yearold male developed nephrotic syndrome with Schnitzler syndrome. A renal biopsy revealed mild thickening of the glomerular basement membrane with spikes and mild expansion of the mesangial matrix; prominent fine granular immunoglobulin G depositions were found along the capillary walls by immunofluorescence study and electron dense deposits were observed in the subepithelial spaces and in a part of mesangium by electron microscopically. The histological findings were compatible with secondary form of membranous nephropathy. To the best of our knowledge this is the first renal biopsy case of Schnitzler syndrome. With corticosteroid treatment chronic rash and proteinuria have disappeared, but immunoglobulin (IgM) paraprotein has been still present.Correspondence to:
Dr. Yoichi Iwafuchi
Department of Internal Medicine
Koseiren Sanjo General Hospital
Tsukanome 5-1-62, Sanjo 955-0055, Japan
Email: [email protected]
Nephrology Education
Reversible left ventricular dysfunction and acute kidney injury in a patient with nonamyloid light chain deposition disease
Nand K. Wadhwa, Amit Kamra, Hal A. Skopicki, Shambavi Richard, Frederick Miller and Edward P. Nord
Page No. 501
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 (501-505)
Reversible left ventricular dysfunction and acute kidney injury in a patient with nonamyloid light chain deposition disease
Nand K. Wadhwa1,4, Amit Kamra1,4, Hal A. Skopicki2,4, Shambavi Richard3,4, Frederick Miller5 and Edward P. Nord1,4
Divisions of 1Nephrology, 2Cardiology, 3Oncology, Department of 4Medicine and Department of 5Pathology, School of Medicine, State University of New York at Stony Brook, Stony Brook, NY, USA
Non-amyloid light chain depositiondisease (LCDD) is a rare entity thatmost commonly presents as proteinuria and/or renal dysfunction. We report on a patientwho initially presented with acutely decompensatedheart failure and subsequently developednephrotic range proteinuria withattendant advanced renal dysfunction. Thediagnosis of LCCD was made on renal biopsy.She was treated with five cycles ofbortezomib and dexamethasone followed bycyclophosphamide priming for peripheralblood stem cell (PBSC) mobilization and autologousstem cell transplant (ASCT). Fouryears later, she remains in very good partialresponse (VGPR) with a left ventricularejection fraction (LVEF) of 58% and serumcreatinine of 1.1 mg/dl. This observationsupports the approach of aggressive managementof patients with LCDD who have multipleorgan failure.Correspondence to:
Nand K. Wadhwa, MD
Division of Nephrology,Department of Medicine,
School of Medicine, HSC T-16 Rm-080
State University of NY at Stony Brook
Stony Brook NY 11794, USA
Email: nand.wadhwa@ sbumed.orgnand.wadhwa@ sbumed.org
Letter to the Editor
Comment on thrombotic microangiopathy induced by interferon-β therapy
Gioacchino Li Cavoli, Rita Passantino, Calogera Tortorici, Luisa Bono, Angelo Ferrantelli and Ugo Rotolo
Page No. 506
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 – Letters to the editor
Comment on thrombotic microangiopathy induced by interferon-β therapy
Gioacchino Li Cavoli1, Rita Passantino2, Calogera Tortorici1, Luisa Bono1, Angelo Ferrantelli1 and Ugo Rotolo1
1Nephrology and Dialysis and 2Pathologic Anatomy, Civic and Di Cristina Hospital, Palermo, Italy
Correspondence to:
Dr. Gioacchino Li Cavoli
Nephrology and Dialysis
Civic and Di Cristina Hospital Palermo
Piazza Nicola Leotta, Palermo, Italy 90100
Email: [email protected]
Letter to the Editor
Castleman disease with preceding symptoms of nephrotic syndrome
Ying Li, Hongyan Tong, Liping Mao, Huiping Wang and Juan Jin
Page No. 508
Abstract
Clinical Nephrology, Vol. 78 – No. 6/2012 – Letters to the editor
Castleman disease with preceding symptoms of nephrotic syndrome
Ying Li1, Hongyan Tong1, Liping Mao1, Huiping Wang2 and Juan Jin2
1Department of Hematology and 2Institute of Nephropathy, The First Affiliated Hospital, Zhejiang University, Hangzhou, China
Correspondence to:
Dr. Hongyan Tong
Department of Hematology
The First Affiliated Hospital
Zhejiang University, #79 Quingchun Road
Hangshou, Zhejiang Province, 31003, China
Email: [email protected]
